A 30s-year-old man was admitted to our hospital with abdominal distension. The physical examination revealed jaundice with right hypochondralgia, and the laboratory data showed high levels of the hepatobiliary enzyme. CT finding showed the cystic dilatation of the common bile duct (CBD) and the gallbladder wall thickness. EUS finding showed sludges in the dilated CBD and a stone in the pancreatic duct. ERCP finding showed two defects in the main pancreatic duct (MPD) . He underwent endoscopic pancreatic sphincterotomy and endoscopic nasopancreatic drainage. Later, he received percutaneous transhepatic gallbladder drainage (PTGBD) to reduce the jaundice. Percutaneous transhepatic cholangiography and nasopancreatography findings showed the cystic dilatation of the CBD to connect to the MPD and two defects in the MPD. He was diagnosed as pancreaticobiliary maljunction with pancreatic stones, and received a successful endoscopic pancreatic stone removal.